Stock

FESA Number

1974

Strain Name

129.C-Tg(Prnp/PRNP)1Drb 129-Prnp<tm1Cwe>/DrbH

Mutation Strain Type

Attribution

Gene/Allele Information

Allele Name Allele MGI ID Gene Name Gene MGI ID Chromosome
Prnp<tm1Cwe> MGI:1888773 Prnp MGI:97769 2

Phenotypic Description

The octameric repeat region of the mouse prion protein was replaced with the hexameric repeat region from the domestic chicken. The mice express mouse PrP at wild-type levels with correct membrane location and orientation but have an altered metal binding region. The mice have been generated to get a better understanding of prion diseases such as CJD in humans.

Orphanet Disorders

OrphaNet ID Name Url
356 Gerstmann-Straussler-Scheinker syndrome http://www.orpha.net/consor/cgi-bin/OC_Exp.php?lng=EN&Expert=356
466 Fatal familial insomnia http://www.orpha.net/consor/cgi-bin/OC_Exp.php?lng=EN&Expert=466
157 941 Huntington disease-like 1 http://www.orpha.net/consor/cgi-bin/OC_Exp.php?lng=EN&Expert=157941
280 397 Familial Alzheimer-like prion disease http://www.orpha.net/consor/cgi-bin/OC_Exp.php?lng=EN&Expert=280397
282 166 Inherited Creutzfeldt-Jakob disease http://www.orpha.net/consor/cgi-bin/OC_Exp.php?lng=EN&Expert=282166

Publications

Displaying 201 - 210 of 226 Stock Publications
Pubmed ID Authors Title Journal
12758067 Curtis J Age-dependent loss of PTP and LTP in the hippocampus of PrP-null mice. Neurobiol Dis (2 003) 13:55-62
22072968 Friedman-Levi Y Fatal prion disease in a mouse model of genetic E200K Creutzfeldt-Jakob disease. PLoS Pathog (2 011) 7:e1002350
10987828 Herms JW Altered intracellular calcium homeostasis in cerebellar granule cells of prion protein-deficient mice. J Neurochem (2 000) 75:1487-92
16135751 Kong Q Chronic wasting disease of elk: transmissibility to humans examined by transgenic mouse models. J Neurosci (2 005) 25:7944-9
9754876 Lipp HP A 2-year longitudinal study of swimming navigation in mice devoid of the prion protein: no evidence for neurological anomalies or spatial learning impairments. Behav Brain Res (1 998) 95:47-54
15951190 Mayer-Sonnenfeld T The metabolism of glycosaminoglycans is impaired in prion diseases. Neurobiol Dis (2 005) 20:738-43
15351724 Nishimura T Cellular prion protein regulates intracellular hydrogen peroxide level and prevents copper-induced apoptosis. Biochem Biophys Res Commun (2 004) 323:218-22
21277354 Ratté S Threshold for epileptiform activity is elevated in prion knockout mice. Neuroscience (2 011) 179:56-61
19073920 Sigurdson CJ De novo generation of a transmissible spongiform encephalopathy by mouse transgenesis. Proc Natl Acad Sci U S A (2 009) 106:304-9
14747574 Tremblay P Mutant PrPSc conformers induced by a synthetic peptide and several prion strains. J Virol (2 004) 78:2088-99

Pages

Orphanet Categories

Rare neurologic disease

EMMA ID

EM:02421

Genetic Status

GMO

Background Strain Name

129

Background Strain MGI ID

MGI:2160041

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